Frontiers in Oncology (Sep 2022)

Case Report: Successful therapy with all-trans retinoic acid combined with chemotherapy followed by hematopoietic stem cell transplantation for acute promyelocytic leukemia carrying the BCOR-RARA fusion gene

  • Li Chen,
  • Hongming Zhu,
  • Yongmei Zhu,
  • Wen Jin,
  • Fangyi Dong,
  • Jianfeng Li,
  • Jiong Hu,
  • Qiusheng Chen,
  • Kankan Wang,
  • Junmin Li

DOI
https://doi.org/10.3389/fonc.2022.1013046
Journal volume & issue
Vol. 12

Abstract

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Acute promyelocytic leukemia (APL) is characterized by the balanced translocation of chromosomes 15 and 17, resulting in the formation of PML-RARA fusion gene. More than 98% of APL have PML-RARA fusion, and less than 2% have other types of RARA gene partners, which named variant APL (vAPL). In the present study, we reported a vAPL with BCOR-RARA, which was the third case of BCOR-RARA APL published. The patient achieved complete remission (CR) with all-trans retinoic acid (ATRA) monotherapy, and molecular CR with ATRA plus standard chemotherapy. After that, he underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) and ATRA maintenance and maintained a molecular CR status. This case provided valuable insights into the accurate identification of vAPL. Moreover, ATRA combined with chemotherapy followed by allo-HSCT was suggested as an optimal choice for those vAPL patients who had a high risk of relapse.

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